Paediatric Cardiology Abhishek Oswal Syllabus Age related changes

Paediatric Cardiology Abhishek Oswal

Syllabus • Age related changes in heart rate and blood pressure: know approximate values for infants and toddlers • Innocent murmurs: definition and how to distinguish from pathological murmurs • Heart failure: symptoms and signs in infants and children, possible causes • Common forms of congenital heart disease: natural history and management of • Acyanotic: ASD, VSD (small, medium and large), PDA, coarctation • Cyanotic: Fallot's, transposition • Infective endocarditis: which children are at risk, preventative measures

Vital signs

Innocent Murmurs • Characteristics • • Soft Systolic a. Symptomatic left Sternal edge No thrills No radiation Otherwise normal heart sounds, no S 3/S 4 • Examples • Still’s murmur – musical, vibratory • Venous hum – through cardiac cycle • Carotid flow murmur – in the neck

Heart Failure • Varied presentation • Dependent on age • Newborn – cyanosis, circulatory collapse, may need NICU • Infant – FTT, sweating, SOB (particularly on feeding) • Child – recurrent chest infections, palpitations, syncope, exercise limitation • Clinical signs: • • • Tachypnoea, Tachycardia Cool peripheries Cardiomegaly Heart murmur, gallop rhythm Hepatomegaly

Causes of HF • Newborn – obstructed outflow tract e. g. • Preductal Co. A • Critical aortic stenosis • Infant – high pulmonary flow e. g. • VSD • Large PDA • Older child – right or left or congestive cardiac failure e. g. • Eisenmenger • Cardiomyopathy

Normal (adult) circulation From lungs From body RA LA RV LV To lungs To body

Defects • Acyanotic • • Atrial septal defect (ASD) Ventricular septal defect (VSD) Persistent ductus arteriosus (PDA) Coarctation of the aorta (Co. A) • Cyanotic • Transposition of the great arteries (TGA) • Tetralogy of Fallot (To. F)

Things to know for each one • Pink or blue? • Presentation • Murmur • Other clinical signs • ECG • CXR • Management

Atrial septal defect From lungs From body RA LA RV LV To lungs To body

Atrial septal defect • Pink • Presentation (often in adulthood): asymptomatic, syncope palpitations, decreased exercise tolerance • 20% of the population • Murmur • Soft systolic murmur ULSE • + (pan systolic murmur if AVSD affecting AV valves) • Fixed split S 2 • ECG • Partial RBBB (RSR’ in V 1) in 90% of ASD • RAD/RVH • Right atrial enlargement - Tall P waves • CXR • Increased pulmonary vascular markings • Rx • Conservative if asymptomatic • Closure age 3 -5 (before starting school) • Cath lab device closure for 80% • Some may require surgical closure if associated with other defects or too large for device

Ventricular Septal Defect From lungs From body RA LA RV LV To lungs To body

Ventricular septal defect • Pink • Presentation: Asymptomatic adult heart failure in an infant • Murmur • Small: blowing pan systolic murmur LLSE, may be associated with a thrill • Large: ? above + rumbling mid diastolic murmur, loud P 2, RV heave • ECG • Large: biventricular hypertrophy by age 2/12, peaked P waves (LA hypertrophy) • CXR • Large: increased pulmonary vascular markings, cardiomegaly, ? pulmonary oedema • Rx • Conservative if asymptomatic. Many close spontaneously before 2 years of age • Large: diuretics, captopril, calories so ready for surgical closure at 3 -5/12

(Eisenmenger syndrome) From lungs From body RA LA RV LV To lungs To body

(Eisenmenger syndrome) • Irreversible conversion of a L R shunt disease into a R L shunt, cyanotic disease due to pulmonary hypertension and RV hypertrophy • Blue • Presentation: cyanosed teen/early 20 s, SOB, reduced exercise tolerance, fatigue, HF, erythrocytosis • CV exam • Clubbing • RV heave, Raised JVP • PSM of VSD disappears as the shunt reverses. May have early diastolic murmur of pulmonary regurgitation • ECG • Right heart hypertrophy (right axis deviation) • CXR • Dilated pulmonary arteries, normal pulmonary vascular markings, normal heart size • Rx • Supportive therapy, night time O 2 • Pulmonary vasodilators e. g. prostaglandin analogues, endothelin antagonists, sildenafil • Only definitive is heart lung transplant

Persistent ductus arteriosus From body From lungs RA LA RV LV To lungs To body

Persistent ductus arteriosus • Pink • Presentation: asymptomatic, recurrent LRTI, FTT (rarely HF) • CV exam • Continuous machinery murmur left infraclavicular region • Bounding pulses • ECG • Normal, LVH • CXR • Normal, increased pulmonary markings • Rx • • Conservative – wait and let it close Medical – NSAIDs e. g. indomethacin, aspirin Small – cath lab closure with coil/plug at 1 year Large – surgical ligation age 1 -3 mo

Coarctation of the aorta From lungs From body RA LA RV LV LA LV RV To lungs To body

Coarctation of the aorta (post ductal) • • Pink Adult type (rare) Presentation: asymptomatic progressive hypertension, resistant to drugs CV exam • • Ejection systolic murmur, upper sternal edge Continuous rumbling murmur on auscultation of the back Radiofemoral delay Upper limb hypertension, lower limb hypotension (difference >20 mm. Hg) • ECG • LVH • CXR • rib notching • 3 sign – visible notch of coarctation in descending aorta • Rx • Cath lab angioplasty/stenting age 3 -5 if detected early and no HF as reduces long term risk of HTN • Surgical repair in adulthood if detected late

Coarctation of the aorta (pre ductal) • Emergency! • Blue grey • Presentation • • May be detected antenatally Collapsed, cyanosed, shocked within the first 2 weeks of life Absent femoral pulses O 2 sat of right arm >> left arm >> feet • Murmur • None • ECG + CXR: likely normal, ? cardiomegaly + HF • Rx • Emergency! • Admit to NICU for ? ventilation, PGE 1 infusion, transfer to cardiac centre • Surgery within 24 hr to resect coarcted segment

Any questions so far?

Transposition of the great arteries From lungs From body RA LA RV LV To body To lungs

Transposition of the great arteries • Emergency! • Blue • Presentation • Should be diagnosed antenatally • Collapsed, shocked, very blue • Murmur • None • Single loud S 2 • ECG • Normal • CXR • Egg on side • Increased pulmonary vascular markings • Rx • Antibiotics + PGE 2 infusion • Catheter atrial septostomy • Corrective surgery at 1 -2/52

Tetralogy of Fallot From lungs From body RA LA RV LV To lungs To body

Tetralogy of Fallot • Malformation of the outflow tract septum leading to • • VSD Overriding aorta Pulmonary stenosis Right ventricular hypertrophy • Blue • Presentation • • Antenatal detection Severe cyanosis at birth Older children with reduced exercise tolerance, squatting on exercise Tet (hypercyanotic) spells

Tetralogy of Fallot • Murmur • Harsh ejection systolic murmur ULSE • ECG • Normal at birth • RVH when older • CXR • Small, boot shaped heart • Pulmonary bay (reduced flow through pulmonary artery) • Reduced pulmonary vasculature • Rx • Surgical (BT) shunt in neonate if severely cyanosed • Aim for elective repair at 6 -9/12 • Tet spells – if >15 min need: intubation + ventilation, fluids, IV pain relief, beta blocker

Summary flow diagram Cyanotic? Yes No Murmur? Yes PSM LLSE (± thrill) No Femoral pulses? To. F Yes TGA VSD No Co. A (preductal) ESM ULSE + fixed split S 2 ASD Continuous machinery PDA ESM back (UL vs LL BP) Co. A (postductal)

Syndromic associations • Down’s • VSD/AVSD (30%) • Turner’s • Bicuspid aortic valve, Coarctation • Fragile X • Mitral valve prolapse, mitral regurgitation, aortic regurgitation • Williams’ • Supravalvular aortic/pulmonary stenosis

Infective Endocarditis • At risk? • Any congenital heart defects, particularly Co. A, VSD, PDA • Any surgical implants, coils, devices, replacement valves • Consider in anyone with a persistent fever/ESR • Preventative measures • Good dental hygiene • Avoid piercings/tattoos/IVDU • No need for prophylactic Abx in dental/any other surgical procedures

MCQ 1 • An uncomplicated VSD in a 5 -year-old boy may be associated with which one of the following? • A. A collapsing pulse • B. Wide and fixed splitting of the second heart sound • C. Clubbing of the fingers • D. A pansystolic murmur of grade 4/6 in intensity • E. Splenomegaly

MCQ 2 • A four year old child is found to have a continuous machinery murmur heard loudest below the left clavicle. He is underweight for age but otherwise well. Considering the likely diagnosis, which of the following would you recommend for this patient? • A. Recommend early operative closure • B. Review the child constantly, expecting spontaneous closure within the next five years • C. Recommend prophylactic penicillin until operation is performed • D. Delay operation until the child has reached its expected weight for age • E. Explain to the parents that this is of little significance and can be ignored

MCQ 3 • A previously well 3 -day-old becomes unresponsive and dusky on the neonatal unit. A CXR shows an “egg on side” appearance of the heart. Which of the following 3 are correct? • A. Femoral pulses may be palpable • B. ECG will show RV hypertrophy • C. There is a loud, single second heart sound • D. There is a ejection systolic murmur at the ULSE • E. Give an immediate prostaglandin infusion

Any questions?

References • RCH, Melbourne heart defects: http: //www. rch. org. au/cardiology/heart_defects/ • Lissauer’s Illustrated textbook of Paediatrics • Beattie, Champion. Essential revision notes in Paediatrics for the MRCPCH • Medscape